Case series of the anaesthesia management in two children with Desbuquois Syndrome and review of literature

Abstract

Background: We report the anaesthesia management of two children with Desbuquois syndrome. Both had characteristic features – flat-face, micrognathia, bell-shaped thorax, hyper-flexible joints, clinodactyly, short neck and dwarfism, and genetic testing confirming the syndrome. Less than 50 cases are documented, with only 2 reporting anaesthesia management in children: this case series a first documenting the course of 2 patients over 4 surgical procedures. Cases: Both the patient had challenging airways, managed with endotracheal intubations due to failed LMA placement. We also postulate an element of subglottic stenosis as despite good view of the glottis, the age-appropriate endotracheal tube could not be negotiated, necessitating the use of a smaller tube in both patients. Post-operative high dependency care was needed in both patients for respiratory insufficiency.Conclusion: Anticipate a difficult airway, difficulty in the placement of regional blocks and have low threshold for intensive care following anaesthesia as respiratory insufficiency is the leading cause of fatality.